Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres
Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres
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DOI：
10.1136/ard.2010.144360
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发表时间：
2011-10-01
影响因子：
27.4
通讯作者：
Vonk, M. C.
中科院分区：
文献类型：
--
作者：
Fransen, J.;Popa-Diaconu, D.;Vonk, M. C.
目的系统性硬化症(SSC)与预期寿命显著降低有关。1999年，280名SSC患者建立了一个预测5年生存率的简单预测模型，但该模型尚未在其他患者中得到验证。预后模型的预测在其他患者中通常不太准确，特别是来自其他中心或国家的患者。在欧洲其他中心进行了一项研究，以验证预测SSC患者5年生存率的预后模型。方法建立了2002年前确诊的SSC患者的欧洲多中心队列。根据美国风湿病学会初步分类标准的SSC患者，如果他们被跟踪至少5年或更短时间，如果他们死亡，就有资格参加研究。主要结果是被诊断为SSc后的5年存活率。预先定义的预后模型使用了以下基线变量：年龄、性别、尿蛋白、血沉(ESR)和一氧化碳弥散量(DLCO)。结果共获得1049例患者的数据，其中119例(11%)在确诊后5年内死亡。在这些患者中，85%是女性，确诊时的平均年龄(SD)为50(14)岁，30%的患者被归类为弥漫性皮肤SSC。年龄(OR 1.03)、男性(OR 1.93)、尿蛋白(OR 2.29)、血沉升高(1.89)、DLCO低(OR 1.94)的预后模型受试者操作特征曲线下面积为0.78。509例无危险因素患者中死亡12例(2.2%)，349例有单一危险因素患者中有45例死亡(13%)，168例有两种危险因素患者中有55例死亡(33%)，23例有三种危险因素患者中有7例死亡(30%)。
Objective Systemic sclerosis (SSc) is associated with a significant reduction in life expectancy. A simple prognostic model to predict 5-year survival in SSc was developed in 1999 in 280 patients, but it has not been validated in other patients. The predictions of a prognostic model are usually less accurate in other patients, especially from other centres or countries. A study was undertaken to validate the prognostic model to predict 5-year survival in SSc in other centres throughout Europe.Methods A European multicentre cohort of patients with SSc diagnosed before 2002 was established. Patients with SSc according to the preliminary American College of Rheumatology classification criteria were eligible for the study when they were followed for at least 5 years or shorter if they died. The primary outcome was 5-year survival after diagnosis of SSc. The predefined prognostic model uses the following baseline variables: age, gender, presence of urine protein, erythrocyte sedimentation rate (ESR) and carbon monoxide diffusing capacity (DLCO).Results Data were available for 1049 patients, 119 (11%) of whom died within 5 years after diagnosis. Of the patients, 85% were female, the mean (SD) age at diagnosis was 50 (14) years and 30% were classified as having diffuse cutaneous SSc. The prognostic model with age (OR 1.03), male gender (OR 1.93), urine protein (OR 2.29), elevated ESR (1.89) and low DLCO (OR 1.94) had an area under the receiver operating characteristic curve of 0.78. Death occurred in 12 (2.2%) of 509 patients with no risk factors, 45 (13%) of 349 patients with one risk factor, 55 (33%) of 168 patients with two risk factors and 7 (30%) of 23 patients with three risk factors.Conclusion A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study.